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Biochemical and immunocytochemical analysis in chronic proximal spinal muscular atrophy
Author(s) -
Hamida Christiane Ben,
SoussiYanicostas Nadia,
ButlerBrowne Gillian S.,
Bejaoui Khemissa,
Hentati Fayçal,
Hamida Mongi Ben
Publication year - 1994
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880170407
Subject(s) - myosin , immunocytochemistry , spinal muscular atrophy , gene isoform , atrophy , biology , muscle atrophy , immunohistochemistry , myh7 , anatomy , pathology , medicine , endocrinology , myosin light chain kinase , microbiology and biotechnology , disease , biochemistry , gene
Immunocytochemical and biochemical analyses were carried out on patients affected by chronic SMA. Three groups of patients were identified. In group I, the muscle presented a fascicular atrophy; a high percentage of atrophic type II fibers; and fibers expressing fast, slow, embryonic, and fetal myosin isoforms. In group II, the muscle was characterized by atrophic fibers and normal/hypertrophic fibers expressing only slow myosin isoforms. In group III, the muscle was characterized by fiber type grouping and fibers coexpressing fast and slow myosin isoforms but never embryonic or fetal MHC isoforms. The muscles of groups I and III contained both fast and slow myosins whereas group II muscles were predominantly slow by immunocytochemical analysis or only slow by biochemical analysis. In view of these results, immunocytochemical and histochemical analyses could help to classify chronic SMA and help to understand the different pathogenic processes which seem to be related to the maturational stage of the muscle at the age of onset of the disease. © 1994 John Wiley & Sons, Inc.